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Mutant ACVR1 arrests glial cell differentiation to drive tumorigenesis in pediatric gliomas

Abstract:

Diffuse intrinsic pontine gliomas (DIPGs) are aggressive pediatric brain tumors for which there is currently no effective treatment. Some of these tumors combine gain-of-function mutations in ACVR1, PIK3CA, and histone H3-encoding genes. The oncogenic mechanisms of action of ACVR1 mutations are currently unknown. Using mouse models, we demonstrate that Acvr1 G328V arrests the differentiation of oligodendroglial lineage cells, and cooperates with Hist1h3b K27M and Pik3ca H1047R to generate hig...

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Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1016/j.ccell.2020.02.002

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Publisher:
Elsevier Publisher's website
Journal:
Cancer Cell Journal website
Volume:
37
Issue:
3
Pages:
308-323
Publication date:
2020-03-05
Acceptance date:
2020-02-04
DOI:
EISSN:
1878-3686
ISSN:
1535-6108
Language:
English
Keywords:
Pubs id:
1093282
Local pid:
pubs:1093282
Deposit date:
2020-03-12

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